1. The orthopaedic features of 100 patients with the Ehlers-Danlos syndrome are described. 2. The significance of these findings is discussed and comment is made of their relationship to the other stigmata of the syndrome.

No abstract

Summary The osteopetroses are a group of conditions which are characterized by varying combinations of bony sclerosis and modelling defects. Classical osteopetrosis may be inherited as an autosomal dominant or autosomal recessive: the former variety is benign, heterogeneous and comparatively common, while the latter is precocious, potentially lethal and rare. Many other craniotubular dysplasias and hyperostoses are loosely grouped with the osteopetroses. The commonest of these is the autosomal dominant form of craniometaphyseal dysplasia, while the others which are well known include Pyle disease, and van Buchem disease. Sclerosteosis is a progressive condition in which massive cranial thickening is associated with syndactyly and gigantism. Each of these disorders has specific clinical and radiographic features, which permit recognition. Diagnostic accuracy is crucial for treatment, prognostication and effective genetic management.

There has been considerable discussion as to the influence of obesity on the indications for, and the outcome after, joint replacement. Attempts have been made to withhold funding for such procedures in those who are overweight. What is the justification for this? This editorial examines the current evidence concerning the influence of obesity on joint replacement and suggests that it is only in the morbidly obese, with a body mass index > 40 kg/m(2), that significant contraindications to operation are present.

Two individuals with generalised articular hypermobility are described. There are many affected members in both kindreds, and the pedigrees indicate that the disorder is transmitted as an autosomal dominant trait. Orthopaedic complications and deformities are common in one family but absent in the other. It is suggested therefore that the two disorders are distinct and separate genetic entities.